POEMS syndrome is a rare multisystem disorder that affects multiple organs in the body. It is characterized by the presence of a plasma cell dyscrasia, which is an abnormality in the plasma cells that produce antibodies. The acronym "POEMS" stands for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.
Due to its rarity, the prevalence of POEMS syndrome is not well-established. However, it is estimated to affect approximately 1 in every 100,000 individuals. The syndrome predominantly occurs in adults, with a slight male predominance. It is more commonly diagnosed in individuals aged 30 to 60 years.
POEMS syndrome is considered a paraneoplastic syndrome, meaning it is associated with an underlying plasma cell disorder, typically multiple myeloma. The exact cause of the syndrome is not fully understood, but it is believed to involve the overproduction of certain cytokines and growth factors by the abnormal plasma cells.
Diagnosis of POEMS syndrome involves a combination of clinical evaluation, laboratory tests, imaging studies, and nerve conduction studies. Treatment options include addressing the underlying plasma cell disorder, managing symptoms, and providing supportive care.